BASIC FACTS ABOUT
JUVENILE PILOCYTIC
ASTROCYTOMA (JPA)
“Where hope
springs eternal”
Pediatric Brain Tumor Foundation
A Resource for Families
ACKNOWLEDGEMENTS
The Pediatric Brain Tumor Foundation wishes to thank
Henry S. Friedman, MD, The Brain Tumor Center, Duke
University Medical Center, Durham, NC, and Ian F.
Pollack, MD, Department of Neurosurgery, Children’s
Hospital of Pittsburgh, Pittsburgh, PA, for scientific review
of this publication.
SOURCES
Statistical data in this publication obtained from the
Central Brain Tumor Registry of the United States
(CBTRUS).
DISCLAIMER
The Pediatric Brain Tumor Foundation does not
engage in rendering medical advice or professional
medical services. Information contained in this
publication is NOT intended to be a substitute for
medical care and should not be used for the diagnosing
or the treatment of a brain tumor or any other health
problem. If you have or even suspect you have a
problem concerning your health or that of someone
else, you should consult with your healthcare provider.
The materials provided by the Pediatric Brain Tumor
Foundation are compiled based on current information
at the time that they were written. Medical research
concerning disease and treatments is an ongoing
process. We endeavor to keep our materials current.
However, you should review with your doctors and
medical institutions to attempt to seek the most current
information available.
COPYRIGHT
Copyright© 2000 by the Pediatric Brain Tumor
Foundation. Reprinted in 2004.
The contents of this publication have been prepared
for the exclusive use of the Pediatric Brain Tumor
Foundation. It may not be reproduced in part or in its
entirety without the written permission of the Pediatric
Brain Tumor Foundation.
Mission Statement
Find the cause and cure of childhood
brain tumors through the support of
medical research;
Increase public awareness about the
severity and prevalence of childhood
brain tumors;
Aid in early detection and treatment
of childhood brain tumors;
Support a national database on all
primary brain tumors; and
Provide hope and emotional support
for the thousands of children and
families affected by this life-threatening
disease.
Pediatric Brain Tumor Foundation
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INTRODUCTION
The news that a child has a brain tumor is nearly
impossible to absorb. Naturally, when parents first
receive such news, they are stunned. Gradually, the
initial shock is replaced by a desire to learn more about
the tumor: Exactly where is it? Which areas of the body
is it affecting? What can be done about it? What lies
ahead? Almost instinctively, people realize that seeking
the answers to these questions will restore a sense of
control.
To help you in your efforts to gain understanding
about your child’s illness, the Pediatric Brain Tumor
Foundation (PBTF) has put together this booklet. It
contains information about the specific type of tumor
that has invaded your family’s world. We encourage you
to learn as much as you can. Trust your instincts. When
you have questions or concerns, voice them. Take an
active part in selecting which approach will result in the
best care for your child.
Pediatric brain tumors require specialized treatment
methods that are most often provided at comprehensive
pediatric hematology/oncology centers by a
multidisciplinary team of pediatric cancer specialists. this
team usually includes physicians, nurses, social
workers,child life specialists, psychologists and
rehabilitation specialists. Such care should provide
families with access to education and support services.
We have much to learn about childhood brain
tumors. Funding research to find causes and cure,
increasing public awareness, and aiding in the early
detection and treatment of childhood brain tumors are
all ways the PBTF fulfills its mission. Families like yours
are at the heart of our mission. If you need further
information or other PBTF publications, our National
Family Support Program Coordinator is on staff to take
your call.
Contact us at 1-800-253-6530 or e-mail:
[email protected].
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Pediatric Brain Tumor Foundation
A FEW BASIC FACTS ABOUT BRAIN
TUMORS
What causes a brain tumor?
The short answer is: We don’t know. Researchers
believe that inherited and genetic factors may be
involved. Environment may play a part. While studies
have yielded valuable information, they have not
provided a concrete answer about the cause. The key
may even lie in areas not yet investigated by science.
What does it mean when a tumor is
classified as Grade I, Grade II, Grade III, or
Grade IV?
Tumors are graded based on their appearance when
examined under the microscope to help determine the
best treatment approach. The more aggressive and
dangerous a tumor is, the higher the grade it is
assigned. Some types of tumors, such as
medulloblastoma, are always considered high grade.
Grade I is considered benign (non-cancerous). Grade
II is a significant step higher with cells that more easily
become malignant, although this is uncommon in
children. Malignant tumors invade and destroy healthy
tissue. Grade III and IV tumors are malignant with
increasing severity.
What is staging?
The “stage” of a cancerous tumor is based on its size
and the extent to which it has metastasized, or spread,
beyond the site of its origin. Because a juvenile pilocytic
astrocytoma (JPA) is benign and generally does not
spread to other sites, physicians usually do not assign a
stage to this type of tumor.
cerebrum
thalamus
(third ventricle)
hypothalamus
posterior fossa
brainstem
midbrain
pons
medulla oblongata
fourth ventricle
cerebellum
Pediatric Brain Tumor Foundation
tentorium
3
JUVENILE PILOCYTIC ASTROCYTOMA (JPA)
What is Juvenile Pilocytic Astrocytoma?
An astrocytoma is a tumor of the glial cells, or
neuroglia, also called “nerve glue.” Glial cells have
different functions: some respond to infection or
damage in the nervous system, while others connect
and support the neurons. Glial cells are involved in a
high percentage of brain and spinal cord tumors.
According to the World Health Organization’s
classification system, a juvenile pilocytic astrocytoma,
often abbreviated JPA, is a grade I tumor. The word
“pilocytic” means “comprised of fiber-shaped cells.”
Well-defined, slow-growing, and usually benign, JPAs
can arise from the cerebellum, cerebrum, or spinal cord.
They have also been found in the structures of the eye,
hypothalamus, thalamus, and the region of the third
ventricle. Ventricles are hollow areas in the brain that
contain cerebrospinal fluid (CSF). The third ventricle is
surrounded by the thalamus.
Although JPAs are typically non-cancerous, that does
not mean they are harmless. Even a benign tumor can
be life-threatening if it is difficult or impossible to reach
safely.
What are the symptoms of a JPA?
Symptoms depend on the exact location of the
tumor. The cerebellum, where JPAs often start, aids in
coordinating voluntary movements and maintains
balance and muscular tone. These are the functions
most affected by a tumor in that area. A JPA in the
cerebrum is likely to cause headaches, nausea, vomiting,
weakness of arms or legs, visual disorders, or seizures. If
the hypothalamus or pituitary gland is involved, effects
may include early onset of puberty, delayed
adolescence, short stature with a decreasing rate of
growth, or behavioral changes. Other indications that a
JPA may be present include diabetes insipidus and
abnormal thinness. If the main bulk of the tumor is in
the cavity of the third ventricle, the flow of CSF may be
blocked, causing hydrocephalus or “water on the brain.”
In babies and very young children whose skull bones
are not firmly set, hydrocephalus can actually cause the
skull to increase in size.
How common are JPAs in children?
JPAs most often occur in the first decade of life, with
the highest incidence being between the ages of five
and nine. Medulloblastoma is the only tumor type more
likely to be found in that age group. JPAs appear to be
equally common in boys and girls. (CBTRUS)
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Pediatric Brain Tumor Foundation
DIAGNOSIS
Diagnosis is the process of finding out which disease
is causing a person’s symptoms. Only after this is done
can the best treatment plan be determined. In many
cases, making the diagnosis of a brain tumor can be
challenging because the early symptoms can mimic
those of other less serious conditions. However, once a
diagnosis of a brain tumor is considered, an imaging
evaluation of the brain is required to confirm the
suspicion. Initially, MRI and CT scans are most useful in
determining the presence of a JPA.
MRI (Magnetic Resonance Imaging)
An MRI scan combines high-frequency radio waves
and a strong magnetic field to produce a picture of the
inside of the body. No x-ray radiation is involved. Before
the test begins, the doctor may inject a special dye
called contrast material into the patient’s vein.
The dye makes it easier to see abnormal tissue. The
procedure usually takes from thirty minutes to an hour.
To have an MRI, the patient lies on a mechanical table.
The table is then moved into a large structure with a
donut-shaped opening. If a “closed MRI” is used, it’s a
little like going into a tunnel. In contrast, an “open MRI”
is open on the top and sides.
Children are never left alone during an MRI. The child
is continually monitored during the procedure. Some
MRI centers will allow parents to remain with their
children as well. Although the scan should not cause
MRI Suite
Pediatric Brain Tumor Foundation
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any pain, the thumping noise that occurs in the
machinery from time to time during the procedure may
startle young children. An accurate scan can be obtained
only if the patient is still. For this reason, a mild sedative
may be used to calm a child who seems very upset. At
times they may play music during the scan to help with
relaxation.
CT (Computerized Tomography)
A CT scan uses a sophisticated x-ray machine
combined with a computer to create a picture of the
inside of the body. Before the test begins, the doctor
may inject a special dye called contrast material into the
patient’s vein. The dye makes it easier to see abnormal
tissue. When it is time for the scan, a technologist
positions the patient on a movable padded table. The
table inches slowly through a donut-shaped scanner
ring, stopping about every half-inch for a picture to be
taken. During the scan, the technologist watches through
a glass window and talks to the patient on an intercom.
The sound of motors and gears can be heard as the
scanner takes the pictures. A CT scan normally takes
less than an hour.
PET (Positron Emission Tomography)
A PET scan provides a picture of brain activity. It may
be used when trying to tell whether a CT or MRI scan is
showing the return of a tumor or tissue damage caused
by radiation. PET may be used in addition to CT or MRI.
SPECT (Single Photon Emission Tomography)
SPECT provides information similar to the PET scan,
but is more widely available.
Biopsy
A biopsy is a procedure in which a sample of tumor
tissue is removed so that doctors can study its
characteristics. The sample can be taken through an
open or surgical biopsy, or a needle biopsy. If the tumor
is difficult or impossible to resect (remove) safely, a
doctor may perform a biopsy to identify the type of
tumor and determine what treatment would be most
useful. If the tumor is surgically resectable, tissue
removed during the resection process can be examined
to confirm the diagnosis.
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Pediatric Brain Tumor Foundation
TREATMENT FOR JPA
Treatment for a JPA often begins with surgery. The
surgeon’s goals are to biopsy the tumor and to resect
(remove) as much of the tumor as possible with
minimal harm to the patient. Because a JPA is usually a
well-defined, distinct capsule, complete removal is
achieved in many cases. However, if the tumor has
grown into an area that is difficult or impossible to reach
safely, such as the brain stem, radiation therapy (RT) or
chemotherapy may be used following surgery to destroy
the remaining tumor cells. Chemotherapy may be
advised, depending on how much of the tumor has
been removed, the age of the child, the location of the
tumor, and whether the tumor has spread.
Surgery
To remove a JPA, the pediatric neurosurgeon must
first gain access to the brain. This is usually done by
craniotomy or craniectomy. “Cranio” means skull.
“Otomy” means surgical incision. “Ectomy” means
surgical removal. For a craniotomy, the surgeon creates
a “bone flap” in the skull which is removed and put
back in place when the operation is over. For a
craniectomy, the bone is removed in pieces and not
replaced.
During surgery, the doctor may find that the JPA is
blocking the cerebrospinal fluid (CSF) pathway. In most
cases, removing the tumor will correct the problem.
Sometimes, however, even when the CSF pathway is
open, it does not function properly. In that case, the
surgeon may insert a small plastic tube called a
ventriculostomy or external ventricular drain (EVD) to
avoid fluid buildup around the time of surgery.
Later, a more permanent internal shunt may be
inserted to keep the fluid circulating around the brain.
Another way to keep the fluid from building up is to
create a small hole in the floor of the third ventricle (a
third ventriculostomy). Sometimes steroids are given to
reduce swelling before, during, and after surgery.
The brain is a complex, sensitive organ, and doctors
and parents must understand and deal with the risks of
brain surgery. Despite these risks, surgery may be
essential to remove as much of the tumor as possible.
Studies show that two out of every ten children who
have brain surgery may develop serious aftereffects.
Examples include loss of speech, balance difficulties,
hormonal disorders, or growth disorders. These
problems may last for weeks, months, or longer. Speech
therapy, physical therapy, or occupational therapy can
help restore function to its maximum potential.
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Staging
Staging determines if a tumor has spread beyond the
site of its origin and if so, to what extent?
To stage a tumor, the doctor reviews scans taken
before and after surgery, and may also analyze samples
of cerebrospinal fluid (CSF) obtained through a spinal
tap. The extent of surgical removal is also used to stage
a tumor. Surgeons commonly designate surgical
resection as being total or near total, subtotal, partial, or
biopsied only. Total resection means no visible residual
tumor although there are always some tumor cells left
behind. Near total means a small amount (less than
10%) of residual tumor left behind. Subtotal means
50%-90% tumor removal and partial means less than
50% tumor removal. Ask your doctor to more fully
explain the term being used to describe the stage of
your child's tumor.
Doctors usually do not stage a JPA. JPAs generally do
not spread to other sites and staging is not meaningful
in most cases.
Radiation Therapy (RT)
Radiation therapy, sometimes called radiotherapy,
uses x-rays to attempt to destroy tumor cells. RT is
highly effective for JPA, but is used selectively for tumors
that are progressive after surgery or that are symtomatic
but can not be removed surgically due to the extent
and/or location of the tumor. RT is usually not needed if
a JPA is completely removed.
The side effects of RT vary from child to child and are
most related to the dose received by the brain and the
age of the child. Radiation effects can be temporary or
permanent, occurring during radiotherapy or not until
months or years later.
Problems that can occur during radiation therapy
include fatigue, nausea or diminished appetite, skin
changes (like sunburn), hair loss, or soreness
swallowing. RT can cause low white blood cell counts
(leaving the child susceptible to infection) or low levels
of platelets (important for clotting and healing). Your
radiation oncologist can often suggest ways to relieve
some of the symptoms seen during radiotherapy.
Later effects of RT include:
RT to the entire brain can cause learning problems
noted several years after therapy. The effects of
irradiation on learning or on memory are quite variable,
but in general are more pronounced when higher doses
are used in younger children. When the disease
presentation permits lower radiation doses to the brain,
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Pediatric Brain Tumor Foundation
the likelihood of encountering later learning difficulties
and the degree of the deficits are both diminished.
Changes in growth can result from direct effects on
spinal bone growth or more commonly from a
reduction in growth hormone. Growth hormone, or GH,
is an important substance produced in the pituitary
gland. GH is often diminished when measured a year or
more after radiation therapy. In a child without evidence
of disease, growth hormone can be replaced as a
medicine.
Changes in other hormones, including thyroid
hormone and sometimes the hormones responsible for
sexual maturation during puberty, can be noted at or
beyond the one-year post-therapy point. When hormone
levels are inadequate, the hormones can be replaced
with medication.
Chemotherapy
Chemotherapy is the use of a single medicine or
combination of medicines to attack tumor tissue.
Chemotherapy can be taken by mouth, by injection, or
through an intravenous line (IV). Some therapies can be
given on an outpatient basis, while others require one
or more days in the hospital.
A JPA may be treated with chemotherapy if the
tumor cannot be completely resected (removed)
surgically and the disease seems to be progressing.
Chemotherapy may be particularly useful if the doctor
wishes to delay or avoid RT.
If your child requires chemotherapy, it is helpful to
keep the following two facts in mind: (1) children do
not normally experience all possible side effects, and
(2) there are ways to relieve or offset many of them.
Among the side effects caused by chemotherapy are
hair loss, skin problems, nausea, vomiting, diarrhea,
constipation, changes in taste, fatigue, mouth sores,
seizures, hearing loss, kidney and liver problems, and
reduction of platelets, red cells, or white cells in the
blood. Remember: some of these problems may not
occur at all. Some may be temporary, while others may
be permanent.
Research is ongoing in an attempt to improve the
effectiveness and reduce the negative side effects of RT
and chemotherapy. If you are interested in learning
about clinical trials or studies that are testing new
approaches, ask your doctor, contact the National
Cancer Institute at http://www.cancer.gov, call the
PBTF at 1-800-253-6530, or e-mail:
[email protected].
Pediatric Brain Tumor Foundation
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PROGNOSIS AND OUTCOME
The likely outcome, or prognosis, for a child with a
brain tumor depends on many factors, including:
The age of the child.
The child’s overall health.
How much the tumor has already affected the
child’s ability to function.
Degree of metastasis, especially the extent to which
the tumor has already spread when first found.
Degree of surgical resection.
The type of treatment received.
When a JPA is completely resected, long-term results
are excellent and complete cures are possible without
RT or chemotherapy. Relative survival rates are currently
95 percent at two years, over 90 percent at five years,
and over 88 percent at ten years in people under age
19 at the time of diagnosis. (CBTRUS)
WHEN A TUMOR RETURNS
The majority of JPAs that have been completely
resected do not recur. Nevertheless, regular follow-up
with MRI or CT scans is recommended for several years
following surgery. If only part of the tumor is removed
(as in near total, subtotal, or partial resection),
recurrence is more likely. If tumor progression is
discovered, RT or chemotherapy may be recommended.
IN THE LONG TERM
More children than ever are surviving brain tumors.
Having survived, however, they and their families must
often deal with long-term effects caused by the tumor
or by the therapy used to treat the tumor.
Among the possible issues are:
decreased levels of growth hormone
decreased levels of thyroid hormone
delayed growth
reduced ability to think and reason
impaired vision or hearing
physical disabilities
fatigue
depression
personality and attitude changes
hydrocephalus
seizures
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Pediatric Brain Tumor Foundation
As distressing as these side effects can be, it is
important to remember that untreated children have
little chance of survival. The choice to do what it takes
to extend life carries with it a choice to accept the risk
that complications may occur. Your best defense is a
good offense. Educate yourself. Seek out other people
who are going through similar experiences, or who have
already been down the road you are traveling. Be
prepared. Don’t hesitate to ask questions, express
concerns, and seek more explanation when needed.
Many of the problems caused by brain tumors or
treatment can be offset. Hormone Replacement
Therapy (HRT) is used to overcome hormone
deficiencies. Physical therapy and occupational therapy
are helpful to children with impaired coordination. If a
child has difficulty talking, speech therapy may be
useful. Specialists can address vision and hearing
problems. The whole family can benefit from social
support programs, including individual, couples or group
counseling. The Pediatric Brain Tumor Foundation offers
Family Support Programs. For more information on
family support programs offered by the Pediatric Brain
Tumor Foundation call (800) 253-6530 or e-mail:
[email protected].
Even if there are no apparent complications, a child
who has had a brain tumor must be monitored closely.
Regular MRI or CT scans may be recommended by
your doctor for several years following surgery. Your
doctor may also recommend hormone evaluation and
neuropsychological testing. Talk with your doctor about
post-treatment monitoring of your child’s tumor.
Your child may sustain late effects from the JPA itself
and/or from treatments for the disease. After
completing treatment, there will be concern for
appropriate reintegration into school. This will require
school placement and interventions to deal with the
child’s particular learning needs. It is important for
parents to work with educators, the medical care team,
and the child to identify individual needs, which can
then be addressed with an Individualized Education
Plan (IEP). Information is available about this process
from the PBTFUS at (800) 253-6530 or e-mail:
[email protected].
Ongoing interaction will help to assure that your
child’s needs are addressed. Consultation with a
neuropsychiatrist or neuropsychologist may be helpful
in evaluating learning needs.
Pediatric Brain Tumor Foundation
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CONCLUSION: HOPE FOR TODAY AND
TOMORROW
By now, you have probably discovered that when you
are dealing with a brain tumor, you do not travel in a
straight line from beginning to end. Instead, one step
forward can be followed by two steps backward,
followed by three steps forward, and so on. An attempt
to treat one complication may create other problems,
which then must be solved. Yet you also experience
small triumphs along the way, and you are always aware
that a major victory may be just around the corner.
As you continue on this difficult journey, we
encourage you to ask questions and express your
concerns. At the same time, seek out people and places
that give you energy and hope. Only when you are
nourished can you give of yourself to those who need
you.
We are making progress against juvenile pilocytic
astrocytoma. Every study and clinical trial increases our
understanding of this tumor’s origin and behavior,
improving our ability to destroy it. One day, we will learn
how to prevent it. And with each advance, more and
more children will be able to enjoy a long and
productive life.
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Pediatric Brain Tumor Foundation
Pediatric Brain Tumor Foundation
Reference Library
The following resource literature and archived Internet
Conference audio compact discs are available to patient families,
medical professionals and social services specialists at no charge
by calling (800) 253-6530 or by e-mailing
[email protected].
1. Questions For Your Medical Care Team When Your Child Has a
Brain Tumor
2. Basic Facts About Pediatric Brain and Spinal Cord Tumors
3. Basic Facts About Medulloblastoma/PNET
4. Basic Facts About Juvenile Pilocytic Astrocytoma
5. Basic Facts About Astrocytoma
6. Basic Facts About Glioma
7. Basic Facts About Ependymoma
8. Helping Hand National Newsletter
9. Caring Hand National Newsletter
10. Informed Parent Internet Series - The Importance of a
Multi-Disciplinary Approach to Treating Children with Brain Tumors
11. Informed Parent Internet Series - The Clinical Trials Process
12. Informed Parent Internet Series - School Re-entry Following the
Diagnoses and Treatment of Your Child’s Brain Tumor
13. Informed Parent Internet Series - Healing the Family
14. Informed Parent Internet Series - Growth and Development:
Endocrine Issues Facing Pediatric Brain Tumor Survivors
15. Informed Parent Internet Series - Post Traumatic Stress: Helping
Families Survive Childhood Cancer
16. Informed Parent Internet Series - Siblings Issues: The Impact of
Cancer on Healthy Siblings
17. Informed Parent Internet Series - Brothers & Sisters & Brain
Tumors: A Child’s Point of View of Coping with Cancer in
the Family
18. Informed Parent Internet Series - Combining Curative and
Pallative Care for Children with Brain Tumors
Pediatric Brain Tumor Foundation
302 Ridgefield Court
Asheville, NC 28806
(828) 665-6891 • (828) 665-6894 (fax)
(800) 253-6530
e-mail
[email protected]
[email protected]
[email protected]
[email protected]
website http://www.pbtfus.org